[Rapid efficacy of preoperative chemotherapy in nephroblastoma involving cardiac cavities].

Archives francaises de pediatrie Pub Date : 1993-12-01
H Carla, C Schoepfer, J R Lusson, C de Riberolles, G Vanneuville, R Goddon, J L Kemeny, P Dechelotte, M Meyer, G Malpuech
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Abstract

Background: Tumors involving the inferior vena cava and cardiac cavities are rare in nephroblastoma. When they do occur, the standard treatment is primary surgery; but this is often technically difficult.

Case report: A 3 year-7-month-old girl was admitted because of an abdominal mass and signs of heart failure. Ultrasonography showed that this mass involved the right renal vein and the inferior vena cava including the right atrium. A CT scan showed no metastases. Echocardiography showed that the mass occupied two thirds of the right atrium and had blocked the tricuspid valve. The patient was given heparin (2 mg/kg/day), vincristine (1.5 mg/m2 on day 1) and actinomycin D (15 micrograms/kg on day 1 to 3). The heart failure disappeared and the abdominal mass decreased in volume. This improvement was confirmed by successive ultrasonographies. Chemotherapy was then completed and the patient was operated on six weeks later: right nephrectomy and removal of the cavoatrial tumoral thrombus. Pathological examination confirmed the nephroblastoma and the patient was given radiotherapy and chemotherapy for 28 weeks. She was also given aspirin for 17 weeks. Pulmonary metastases were detected 1 year after onset and were treated by a combination of surgery and chemotherapy. The patient is well 20 months after the onset of the disease.

Conclusion: Preoperative chemotherapy seems to be effective in nephroblastoma extending to cardiac cavities; it allows subsequent surgery and facilitates postoperative radiotherapy.

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[术前化疗治疗累及心腔的肾母细胞瘤的快速疗效]。
背景:肿瘤累及下腔静脉和心腔在肾母细胞瘤中是罕见的。当它们确实发生时,标准的治疗是初级手术;但这通常在技术上很困难。病例报告:一名3 -7个月大的女婴因腹部肿块和心力衰竭而入院。超声检查显示该肿块累及右肾静脉和下腔静脉,包括右心房。CT扫描未见转移。超声心动图显示肿块占据了三分之二的右心房,阻塞了三尖瓣。患者给予肝素(2 mg/kg/ D)、长春新碱(1.5 mg/m2,第1天)、放线菌素D(15微克/kg,第1 ~ 3天)治疗,心力衰竭消失,腹部肿块体积减小。连续的超声检查证实了这种改善。化疗完成后,患者于6周后进行手术:右肾切除术和去除腔房肿瘤血栓。病理检查证实为肾母细胞瘤,给予放疗和化疗28周。她还服用了17周的阿司匹林。发病1年后发现肺转移,采用手术和化疗联合治疗。病人发病20个月后身体健康。结论:术前化疗对肾母细胞瘤扩散至心腔有效;它允许后续手术并促进术后放疗。
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